• Clinical and Experimental Pediatrics
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• 제64권7호
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• pp.355-363
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• 2021

Background: Nephrotic syndrome (NS) is a common renal disorder in children attributed to podocyte injury. However, children with the same diagnosis have markedly variable treatment responses, clinical courses, and outcomes, suggesting molecular heterogeneity. Purpose: This study aimed to explore the molecular responses of podocytes to nephrotic plasma to identify specific genes and signaling pathways differentiating various clinical NS groups as well as biological processes that drive injury in normal podocytes. Methods: Transcriptome profiles from immortalized human podocyte cell line exposed to the plasma of 8 subjects (steroid-sensitive nephrotic syndrome [SSNS], n=4; steroid-resistant nephrotic syndrome [SRNS], n=2; and healthy adult individuals [control], n=2) were generated using microarray analysis. Results: Unsupervised hierarchical clustering of global gene expression data was broadly correlated with the clinical classification of NS. Differential gene expression (DGE) analysis of diseased groups (SSNS or SRNS) versus healthy controls identified 105 genes (58 up-regulated, 47 down-regulated) in SSNS and 139 genes (78 up-regulated, 61 down-regulated) in SRNS with 55 common to SSNS and SRNS, while the rest were unique (50 in SSNS, 84 genes in SRNS). Pathway analysis of the significant (P≤0.05, -1≤ log2 FC ≥1) differentially expressed genes identified the transforming growth factor-β and Janus kinase-signal transducer and activator of transcription pathways to be involved in both SSNS and SRNS. DGE analysis of SSNS versus SRNS identified 2,350 genes with values of P≤0.05, and a heatmap of corresponding expression values of these genes in each subject showed clear differences in SSNS and SRNS. Conclusion: Our study observations indicate that, although podocyte injury follows similar pathways in different clinical subgroups, the pathways are modulated differently as evidenced by the heatmap. Such transcriptome profiling with a larger cohort can stratify patients into intrinsic subtypes and provide insight into the molecular mechanisms of podocyte injury.

• 한국임상약학회지
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• 제10권2호
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• pp.51-56
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• 2000

Deflazacort, an oxazoline derivative of prednisolone, has been claimed to have anti-inflammatory effects with fewer side effects compared to prednisone. The objectives of the study were to evaluate efficacy and safety of deflazacort in children with nephrotic syndrome. Eligible Patients were the children with nephrotic syndrome who were treated with deflazacort from October. 1994 to April. 1999. Nephrotic syndrome was defined as having albumin level of less than 2.5 mg/dL and 24-hour urinary protein excretion of greater than $40\;mg/m^2/hr$. The primary parameters evaluating the efficacy of deflazacort were response rate, time to respond and relapse frequency. The safety profiles were the impact on children's growth, calcium sparing effect, glucose metabolism, lipid profile and adverse drug reactions. As results, total of 60 children were evaluated (47 boys, 13 girls). Response rate was $95\%$ (57/60) for initial and late responders. Median time to respond was 12 days (range 7-110 days) and median relapse frequency was one time (range 0-6). Weight/height ratio increased from $22.05\pm3.47\;to\23.20\pm3.44\;kg/m$ (p<0.001) and plasma calcium level, from $7.55\pm3.86\;to\;9.98\pm3.77\;mg/dL$ after treatment (p<0.001). Change of fasting glucose level was not statistically significant $(91.92\pm3.53\;vs.\;98.19\pm4.78\;mg/dL,\;p=0.072)$, while change of total cholesterol was significant $(362.3\pm12.0\;vs\;251.4\pm11.5\;mg/dL$, p<0.001). In conclusion, patients on deflazacort showed similar efficacy in treatment of nephrotic syndrome as reported for prednisone with less impact on growth inhibition and metabolic side effects of hyperglycemia and hyperlipidemia.

• Clinical and Experimental Pediatrics
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• 제54권8호
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• pp.322-328
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• 2011

Nephrotic syndrome (NS) is one of the most common glomerular diseases that affect children. Renal histology reveals the presence of minimal change nephrotic syndrome (MCNS) in more than 80% of these patients. Most patients with MCNS have favorable outcomes without complications. However, a few of these children have lesions of focal segmental glomerulosclerosis, suffer from severe and prolonged proteinuria, and are at high risk for complications. Complications of NS are divided into two categories: disease-associated and drug-related complications. Disease-associated complications include infections (e.g., peritonitis, sepsis, cellulitis, and chicken pox), thromboembolism (e.g., venous thromboembolism and pulmonary embolism), hypovolemic crisis (e.g., abdominal pain, tachycardia, and hypotension), cardiovascular problems (e.g., hyperlipidemia), acute renal failure, anemia, and others (e.g., hypothyroidism, hypocalcemia, bone disease, and intussusception). The main pathomechanism of disease-associated complications originates from the large loss of plasma proteins in the urine of nephrotic children. The majority of children with MCNS who respond to treatment with corticosteroids or cytotoxic agents have smaller and milder complications than those with steroid-resistant NS. Corticosteroids, alkylating agents, cyclosporin A, and mycophenolate mofetil have often been used to treat NS, and these drugs have treatment-related complications. Early detection and appropriate treatment of these complications will improve outcomes for patients with NS.

• The Korean Journal of Physiology and Pharmacology
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• 제14권4호
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• pp.223-228
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• 2010

The collecting duct endothelin (ET) system, which involves ET-1 and its two receptors, may play a role in the regulation of renal sodium in association with the nitric oxide synthase (NOS) system. We determined whether sodium retention is associated with changes in the endothelin and NOS systems at different stages (i.e., a sodium retaining stage and a compensatory stage) of nephrotic syndromes. On day 7 after puromycin aminonucleoside (PAN) injection, urinary sodium excretion was decreased, ascites had developed, and there was a positive sodium balance. ET-1 mRNA expression was increased in the inner medulla of the kidney, whereas protein expression of ET receptor type B ($ET_BR$) was unchanged. The expression of neuronal NOS (nNOS) was decreased in the inner medulla. On day 14, urinary sodium excretion was unchanged compared with controls. The expression of $ET_BR$ increased, while nNOS expression in the inner medulla was comparable to controls. These findings suggest that decreased nNOS plays a role in the development of sodium retention in the nephrotic syndrome. Recovery of nNOS and increased renal $ET_BR$ synthesis may promote sodium excretion in later stages of the nephrotic syndrome (on day 14).

• Childhood Kidney Diseases
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• 제22권2호
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• pp.91-96
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• 2018

Nephrotic syndrome in the first year of life, characterized by renal dysfunction and proteinuria, is associated with a heterogeneous group of disorders. These disorders are often related to genetic mutations, but the syndrome can also be caused by a variety of other diseases. We report an infant with nephrotic syndrome associated with a neuroblastoma. A 6-month-old girl was admitted with a 10% weight loss over 10 days and nephrotic-range proteinuria. She was ill-looking, and her blood pressure was higher than normal for her age. Her cystatin-C glomerular filtration rate was decreased, and levels of plasma renin, aldosterone, and catecholamines were elevated. Renal ultrasonography and abdominal computed tomography showed a retroperitoneal prevertebral mass encasing both renal arteries and the left renal vein. The mass was partially resected laparoscopically, and the pathologic diagnosis was neuroblastoma. Findings on a simultaneous renal biopsy were unremarkable. The patient was treated with chemotherapy and several anti-hypertensive drugs, including an alpha blocker. Two months later, the mass had decreased in size and the proteinuria and hypertension were gradually improving. In an infant with abnormal renin-angiotensin system activation, severe hypertension, and nephrotic-range proteinuria, neuroblastoma can be considered in the differential diagnosis.

• Child Health Nursing Research
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• 제10권2호
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• pp.188-195
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• 2004

Purpose: This study was to identify and to search the related disposition of the pattern of anxiety and coping in mothers of children with nephrotic syndrome through the use of Q-methodology. Method: 34 Q-samples were finally selected in the concept of anxiety and coping. A P-sample of 35 was selected the mothers of children with nephortic syndrome. The result of the Q-sorting was coded and analyzed using QUANL PC program. Result: There were 3 types of special opinion. The first type is called ' Pursuit of hope type.' Members of this type were cope with the anxiety by spiritual behavior like a pray, positive thinking. The second type is called 'Worry about reality type.' Members of this type were to be filled with apprehension like an indigestion, insomnia. The third type is called ' Solving problem type.' Members of this type were cope with the humanity effort by conversation. Conclusion: The mothers of children with nephrotic syndrome were used various coping patterns to cope with the anxiety conditions that their child were result from admission to hospital and treatment of the disease. Therefore, nursing assessment and nursing intervention skills have to develop in consideration of the subjectivity of coping about anxiety in each individual.

• Tuberculosis and Respiratory Diseases
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• 제46권4호
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• pp.574-579
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• 1999

저자들은 신증후군에 동반된 거대세포바이러스 폐렴 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Childhood Kidney Diseases
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• 제27권1호
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• pp.1-10
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• 2023

Pediatric nephrotic syndrome (NS) is a clinical syndrome characterized by massive proteinuria, hypoalbuminemia, and generalized edema. Most childhood NS cases are idiopathic (with an unknown etiology). Traditional therapeutic approaches based on immunosuppressive agents largely support the key role of the immune system in idiopathic NS (INS), especially in the steroid-sensitive form. Although most previous studies have suggested the main role of T cell dysfunction and/or the abnormal secretion of certain glomerular permeability factors, recent studies have emphasized the role of B cells since the therapeutic efficacy of B cell depletion therapy in inducing and/or maintaining prolonged remission in patients with INS was confirmed. Furthermore, several studies have detected circulating autoantibodies that target podocyte proteins in a subset of patients with INS, suggesting an autoimmune-mediated etiology of INS. Accordingly, a new therapeutic modality using B cell-depleting drugs has been attempted, with significant effects in a subset of patients with INS. Currently, INS is considered an immune-mediated disorder caused by a complex interplay between T cells, B cells, soluble factors, and podocytes, which may vary among patients. More in-depth investigations of the pathogenic pathways of INS are required for an effective personalized therapeutic approach and to define precise targets for therapeutic intervention.

• Tuberculosis and Respiratory Diseases
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• 제65권1호
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• pp.37-40
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• 2008

본 증례는 소세포폐암에 동반된 미세변화 신증후군에 대한 국내 첫 번째 보고이며, 흉막여출액에 의한 호흡곤란과 말초부종 등 미세변화 신증훈군의 증상 발현에 의해 내원하여 잠재암이 발견된 경우이다. 본 증례를 통하여 성인의 경우 막성신병증 뿐만 아니라 미세변화 신증후군이라도 초기진단 시 원인질환으로 종양의 가능성을 고려해야 한다는 것을 경험하게 되었다.

• 한국임상수의학회지
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• 제20권4호
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• pp.431-436
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• 2003

이 연구의 목적은 실험적으로 유발한 면역매개성 사구체 질환이 신증후군으로 진행하는 과정과 치유과정에서 단백뇨 배설에 근거한 조기진단 및 치유과정의 감시에 대한 평가를 하는 것이다. 두당 endotoxin 1 $\mu$g과 native bovine serum albumin 5mg 을 9두의 건강한 개에 정맥주사하여 감작시키고, 그 후 1주일부터 양이온화한 bovine serum albumin 120mg을 주 5회 반복 정맥주사한 결과 5두에서 면역매개성 사구체신염과 신증후군이 발생하였다. 사구체신염이 발생한 실험동물에서는 요단백질/ 크레아티닌 비의 증가 (>1.0), 저알부민혈증 (< g/이), BUN 증가 (>40 mg/dl), 부종 등이 나타났다. 이것은 임상병리학적으로 평가 가능한 면역매개성 사구체시염 및 신증후군 모델견이 제작되었음을 제시하는 것이었다. 사구체신염의 유발과 신증후군의 진행과정에 요단백질/크레아티닌 비의 증가가 저알부민혈증, BUN 증가, 고콜레스테롤혈증보다 1-4주 먼저 출현하였다. 사구체신염에 의해 신증후군이 유발된 실험동물 모두에서 prednisolone 을 2.2 mg/kg씩 1일 2회 경구투여한 결과 혈중 알부민 농도의 증가와 콜레스테롤 농도의 감소가 먼저 일어나고, 그 후 1-4 주에 요단백질/크레아티닌 비가 감소하기 시작하였다. 종합적으로 볼때, 면역매개성 사구체신염성 신증후군의 조기진단에는 요 단백질/크레아티닌 비가 유리한 지표로, 그리고 초기 치유과정의 감시에는 혈청 알부민, 콜레스테롤이 더 유리한 임상 지표로 이용될 수 있음을 확인하였다.